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Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
Open accessAbstract
BACKGROUND: In late 2012, ivacaftor became available in the UK for people with cystic fibrosis (CF) aged 6 years and over with a G551D mutation. Long-term changes in treatment patterns have not previously been reported. We investigated long-term treatment patterns in people with CF with a G551D mutation who took ivacaftor and compared these with non-ivacaftor-treated cohorts using the UK Cystic Fibrosis Registry. METHODS: Using 2007-2018 data we compared treatment patterns between four cohorts: 1: ivacaftor-treated; 2: ivacaftor era (2013-2018), ineligible genotype (no G551D mutation); 3: pre-ivacaftor era (2007-2012), eligible genotype (G551D mutation); 4: pre-ivacaftor era, ineligible genotype. Treatments included: inhaled antibiotics, dornase alfa, hypertonic saline, chronic oral antibiotics and supplementary feeding. RESULTS: Up to 2012 the percentages of people taking each treatment were similar between the two cohorts defined by genotype and tended to increase by year with a similar slope. Once ivacaftor was introduced, the use of other treatments tended to decrease or remain stable by year for the ivacaftor-treated cohort, whereas it remained stable or increased in the non-ivacaftor-treated cohort. This led to differences in treatment use between the two cohorts in the ivacaftor-era, which became more marked over time. CONCLUSIONS: We have shown a clear divergence in treatment patterns since the introduction of ivacaftor in a number of key treatments widely used in CF. Further research is needed to investigate whether the differences in treatment patterns are associated with changes in health outcomes.
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CFTR modulator treatments have changed the landscape of CF care, including the relationship that people with CF and their families may have with their condition and treatments, their patterns of adherence, as well as clinician views and prescribing practices.
“These treatments have changed the landscape of CF care, including the relationship that people with CF (and their families) may have with their condition and treatments, their patterns of adherence, as well as clinician views and prescribing practices (Granger 2022).”
From Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · cites this paper as Granger 2022
The Abstract and Discussion (page not provided) report differences in recorded chronic-treatment patterns among people taking ivacaftor, supporting the point about prescribing practices. The Introduction raises a possible change in patients’ perception of treatment burden, but the paper does not establish changes in family relationships or clinician views; the Discussion explicitly says adherence levels are unknown. (LLM, conf=0.86)
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Update on Key Emerging Challenges in Cystic Fibrosis
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A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., Griese, M., McKone, E. F., Wainwright, C. E., Konstan, M. W., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1105185 · Cited by 2,195
High treatment burden in adults with cystic fibrosis: challenges to disease self-management
Sawicki, G. S., Sellers, D. E., & Robinson, W. M. (2009) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2008.09.007 · Cited by 498
Volkova, N., Moy, K., Evans, J., Campbell, D., Tian, S., Simard, C., Higgins, M., Konstan, M. W., Sawicki, G. S., Elbert, A., et al. (2020) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2019.05.015 · Cited by 258
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Characterising burden of treatment in cystic fibrosis to identify priority areas for clinical trials
Davies, G., Rowbotham, N. J., Smith, S., Elliot, Z. C., Gathercole, K., Rayner, O., Leighton, P. A., Herbert, S., Duff, A. J., Chandran, S., et al. (2020) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2019.10.025 · Cited by 99
Self-citationData Resource Profile: The UK Cystic Fibrosis Registry
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Real-World Outcomes Among Patients with Cystic Fibrosis Treated with Ivacaftor: 2012–2016 Experience
Higgins, M., Volkova, N. V., Moy, K. A., Marshall, B. C., & Bilton, D. (2020) · Pulmonary Therapy · doi:10.1007/s41030-020-00115-8 · Cited by 33
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Dawson, S., Girling, C.-J., Cowap, L., & Clark-Carter, D. (2023) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd013766.pub2 · Cited by 14
Metadata
- Authors
- Granger, E., Davies, G., & Keogh, R. H.
- Affiliations
- London School of Hygiene & Tropical Medicine (GB)
- University College London (GB)
- Corresponding author
- Granger, Emily
- Venue
- Journal of Cystic Fibrosis
- Publisher
- Elsevier BV
- Published
- 2022-03
- Volume
- 21
- Issue
- 2
- Pages
- 316-322
- ISSN
- 1569-1993 (ISSN-L)
- PMC
- PMC9097695
- OpenAlex
- W3170503017
- Cited by
- 21
- References
- 16
- Citations by year
- 2026: 5 · 2025: 5 · 2024: 3 · 2023: 3 · 2022: 5
- FWCI
- 1.78
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Cystic Fibrosis (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- LSHTM Research Online (London School of Hygiene and Tropical Medicine) (acceptedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Medical Research Council (MR/S017968/1, MR/T041285/1)
- National Institute for Health Research
- Cystic Fibrosis Trust
- NIHR Great Ormond Street Hospital Biomedical Research Centre
- UK Research and Innovation
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Inhalation and Respiratory Drug Delivery
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
- Keywords
- ivacaftor, G551D mutation, treatment patterns, inhaled antibiotics, dornase alfa, hypertonic saline, supplementary feeding, CFTR modulator therapy
- MeSH terms
- Aminophenols, Anti-Bacterial Agents, Cystic Fibrosis, Humans, Mutation, Quinolones, Cystic Fibrosis Transmembrane Conductance Regulator
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